Demystifying autoimmune inner ear disease

Das, Soumyajit; Bakshi, Satvinder Singh; Ramesh, S.

doi:10.1007/s00405-019-05681-5

Cited by 32 publications

(74 citation statements)

References 62 publications

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“…AIED is a poorly understood disease first described in 1940 and represents an uncommon cause of SNHL. 3 Similar to that of SLE, the pathogenesis of AIED is hypothesized to involve both proinflammatory T-cell responses and autoantibody formation. Immune-mediated damage to the stria vascularis, spiral ligament, and Organ of Corti result in SNHL in patients with AIED.…”

Section: Discussionmentioning

confidence: 99%

“…These include humoral-type antibody attacks on inner ear antigens, cell-mediated cytotoxic damage to cochlear and vestibular hair cells, and immune-complex deposition in the microvasculature of the inner ear. 2 Similar mechanisms are postulated to cause rapidly progressive, bilateral sensorineural hearing loss (SNHL) in patients with autoimmune inner ear disease (AIED), 3 a poorly described clinical entity first described by McCabe in 1979. 4 Of note, the prevalence of SLE has been observed to be higher in patients with Ménière’s disease.…”

Section: Introductionmentioning

confidence: 97%

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Hearing loss in patients with systemic lupus erythematosus: A systematic review and meta-analysis

Yuen

Fried

Nguyen

et al. 2021

Lupus

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Objective To determine the prevalence of hearing loss (HL) in patients with systemic lupus erythematosus (SLE), describe frequency-specific hearing threshold changes in this patient population as compared to age-matched control, and compare the clinical and serological profiles of patients with SLE with and without HL. Methods A systematic review querying four databases (PubMed, Web of Science, Scopus, and Cochrane) was performed. Meta-analysis of available data was performed to determine the overall prevalence and odds ratio (OR) of HL, and compare mean differences in frequency-specific hearing thresholds between patients with SLE and control. Additionally, meta-analysis of proportions allowed for comparison of disease features present in patients with SLE with and without sensorineural HL. Results This review included 17 studies reporting on 1326 patients (635 with SLE and 691 control). The pooled prevalence of HL in patients with SLE was 27%. In comparison to control, patients with SLE had a significantly higher odds of HL (OR 14.6, 95% CI: 8.5 to 25.0). Mean air-conduction hearing thresholds in patients with SLE were significantly elevated relative to control at 125 and 250 Hz. Mean bone-conduction hearing thresholds were significantly elevated in patients with SLE across all measured frequencies except at 3000 and 6000 Hz compared to control. Disease features did not significantly differ between patients with SLE with and without HL. Conclusion Compared to age-matched control, patients with SLE have increased odds of HL, which primarily manifests at low frequencies. Therefore, this patient population requires greater audiologic attention.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 97%

Hearing loss in patients with systemic lupus erythematosus: A systematic review and meta-analysis

Yuen

Fried

Nguyen

et al. 2021

Lupus

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“…Sensorineural hearing loss (SNHL) has been defined as a fall of at least 30 dB of the hearing threshold in at least three consecutive frequencies [1]. It has an incidence that varies from five to 20 individuals per 100,000 per year [1,2]. Although in 70% to 90% of the cases the etiology is not identified, vascular injury, rupture of membranes, bacterial or viral infection, and immune-mediated injuries have been proposed as mechanisms 1 1 1, 2 1 3 3 1, 2 of disease [1].…”

Section: Introductionmentioning

confidence: 99%

“…AIED usually presents as a subacute, rapidly progressive, often fluctuating, bilateral and asymmetrical SNHL [1][2][3][4]. Almost 80% of the patients have involvement of both ears [2,5].…”

Section: Introductionmentioning

confidence: 99%

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Sensorineural Hearing Loss and Systemic Autoimmune Disease: The Experience of a Systemic Immune-Mediated Diseases Unit

Ribeiro¹,

Serôdio²,

Amaral³

et al. 2021

Cureus

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Background Autoimmune inner ear disease (AIED) represents less than 1% of all cases of sensorineural hearing loss (SNHL) but its frequency may be underestimated due to lack of specific clinical and laboratory criteria. AIED can be associated with a systemic autoimmune disease (SAID) in 15%-30% of the cases. The objective of the present study was to characterize the clinical and prognostic factors of a cohort of patients with AIED. Materials and methods The authors conducted a retrospective descriptive analysis of a cohort of patients with AIED referred from the otorhinolaryngology department to a systemic immune-mediated diseases unit between March 2013 and November 2020. A consecutive sample of 39 patients with suspected AIED was referred. SNHL was defined as a fall of the hearing threshold of at least 30 decibels in three consecutive frequencies. Eight patients were excluded for not meeting the audiometric criteria or having confounding factors. The remaining 31 patients were included with a total of 50 affected ears. To classify the intensity of hearing loss, an arithmetic mean of pure tone was calculated. Normal hearing or mild hearing loss at the last pure tone audiometry of the followup were an indicator of good prognosis and were considered the outcome of interest. Results Thirty-two percent of the patients had an associated SAID. There were no differences regarding demographic and clinical characteristics when comparing patients with AIED alone and patients with AIED and a SAID, except for the positivity of antinuclear antibodies (ANA). ANA positivity was superior in patients with the association of AIED and a SAID when compared with patients with AIED alone (90% vs 50%; p=0.037). The SAID was diagnosed after the AIED in 70% of the patients, in which diagnosis of the SAID occurred a median of 4,2 (IQR 0.8-5.1) years after the diagnosis of the AIED. Normal audiometric evaluation or a mild hearing loss was achieved in 31% of the ears at the last audiometric evaluation. A normal audiometry or a mild hearing loss at the time of diagnosis was independently associated with a better outcome (31%, 14%, CI 1.71-273.69; p=0.018). Bilateral hearing loss was independently associated with a worse outcome (54%, 79%, CI 0.01-0.84; p=0.035). The use of systemic corticosteroids (p=0.941), transtympanic corticosteroids (p=0.700) and non-steroid immunomodulator drugs (p=0.986) did not affect prognosis. The presence of a SIAD did not affect the prognosis (p=0.986). Conclusions In this cohort, SAID was present in one-third of the patients with AIED. A good prognosis was achieved in one-third of the patients. A normal audiometry or mild disease at presentation was associated with a good outcome, whilst bilateral involvement was associated with a bad one. Association of a SAID did not seem to influence the hearing-related prognosis. Positivity of ANA antibodies may justify performing a complementary investigation to determine the presence of a SAID.

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Environmental Risk Factors, Protective Factors, and Biomarkers for Hearing Loss: An Umbrella Review

Xu,

Liao,

Zhang

et al. 2024

Otolaryngol.--head neck surg.

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ObjectiveWe aimed to investigate the potential environmental risk factors, protective factors, and biomarkers of hearing loss (HL), and establish a hierarchy of evidence.Data SourcesEmbase, PubMed, Cochrane Library, and Web of Science electronic database from inception to June 1, 2023.Review MethodsWe included meta‐analyses of observational studies of associations between HL and environmental risk factors, protective factors, or biomarkers. We calculated summary effect estimates, 95% confidence interval, heterogeneity I2 statistic, 95% prediction interval, small study effects, and excess significance biases.ResultsOf the 9211 articles retrieved, 60 eligible articles were included. The 60 eligible articles identified 47 potential environmental risk and protective factors (N = 4,123,803) and 46 potential biomarkers (N = 173,701). Evidence of association was convincing (class I) for rheumatoid arthritis (RA) and every 1 cm increase in height. Evidence of association was highly suggestive (class II) for human immunodeficiency virus (HIV), diabetes, cumulative noise exposure (CNE), smoking, congenital cytomegalovirus (CMV) infection, combined exposure to organic solvents and noise, non‐Gaussian noise exposure, each 1 kg increase in birth weight, noise exposure, and alopecia areata (AA).ConclusionIn this umbrella review, RA, every 1 cm increase in height, HIV, diabetes, CNE, smoking, congenital CMV infection, combined exposure to organic solvents and noise, non‐Gaussian noise exposure, each 1 kg increase in birth weight, noise exposure, and AA were strongly associated with HL.

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Demystifying autoimmune inner ear disease

Cited by 32 publications

References 62 publications

Hearing loss in patients with systemic lupus erythematosus: A systematic review and meta-analysis

Hearing loss in patients with systemic lupus erythematosus: A systematic review and meta-analysis

Sensorineural Hearing Loss and Systemic Autoimmune Disease: The Experience of a Systemic Immune-Mediated Diseases Unit

Environmental Risk Factors, Protective Factors, and Biomarkers for Hearing Loss: An Umbrella Review

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